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原发性纵隔大细胞淋巴瘤

Primary large-cell lymphomas of the mediastinum.

作者信息

Suster S

机构信息

Department of Pathology, The Ohio State University, Columbus 43210, USA.

出版信息

Semin Diagn Pathol. 1999 Feb;16(1):51-64.

Abstract

The mediastinum is a frequent site of involvement for malignant lymphoma. The most common types encountered in this location include Hodgkin's and non-Hodgkin's lymphoma and lymphoblastic lymphoma. With the exception of lymphoblastic lymphoma in children and adolescents, most cases of mediastinal involvement by lymphoma represent part of systemic disease and do not generally pose difficulties for diagnosis. However, a relatively small group of tumors have become increasingly recognized in recent years that are primarily localized in the anterior mediastinum and show features of nonlymphoblastic/non-Hodgkin's large-cell lymphoma. Because such tumors are capable of adopting unusual morphological appearances, they can often pose difficulties for diagnosis and be frequently mistaken for other conditions. This review discusses this group of neoplasms collectively known as diffuse large-cell lymphoma of the mediastinum. The clinicopathologic, immunohistochemical, and molecular genetic aspects of these tumors appear to indicate that a significant proportion of them may correspond to a distinctive type of lymphoproliferative process most likely arising from a native B-cell population of the thymus, thus representing, in essence, a primary extranodal large-B-cell lymphoma of the thymus.

摘要

纵隔是恶性淋巴瘤常见的受累部位。该部位最常见的类型包括霍奇金淋巴瘤、非霍奇金淋巴瘤和淋巴母细胞淋巴瘤。除儿童和青少年的淋巴母细胞淋巴瘤外,大多数纵隔淋巴瘤受累病例是全身疾病的一部分,一般诊断并不困难。然而,近年来越来越认识到有一小部分肿瘤主要局限于前纵隔,表现为非淋巴母细胞/非霍奇金大细胞淋巴瘤的特征。由于这类肿瘤能够呈现不寻常的形态外观,它们常常给诊断带来困难,且经常被误诊为其他疾病。本综述讨论了这组统称为纵隔弥漫性大细胞淋巴瘤的肿瘤。这些肿瘤的临床病理、免疫组化和分子遗传学特征似乎表明,其中很大一部分可能对应于一种独特的淋巴增殖过程,很可能起源于胸腺的原始B细胞群体,因此本质上代表胸腺原发性结外大B细胞淋巴瘤。

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